Despite having had lymphedema since I was an infant, I never actually heard of it until my official diagnosis at age fourteen, and even then I was still pretty confused: how was I supposed to understand something that my own doctors couldn’t identify for over a decade?
I had a tenuous grasp of lymphedema in those early years after my diagnosis, and it wasn’t until I started seeing a lymphedema therapist at GBMC Lymphedema Center that I finally began to comprehend what it was, what it meant to have it, and why treatment is so crucial in its management. For example, I knew that wearing compression garments helped to reduce my swelling, but now I know how they help reduce it (lymph flow is passive—compression helps get it moving!). I knew that practicing good skin care was important, but now I know why it’s so important (dry skin causes cracks and breaks, which invite infection!).
Knowledge is empowering, especially for patients, because when we better understand what’s going on with our lymphatic system, we can better understand how to take care of it. Plus, we can help others better understand it, too!
To understand lymphedema, we first need to understand the lymphatic system.
The lymphatic system is our second circulatory system: it’s responsible for collecting and removing waste products left behind in tissues, such as fluid and debris, pathogens, and some cancer cells. The clear, protein-rich fluid that carries this waste is called lymph, and it contains plasma and lymphocytes (white blood cells), which fight infections and the growth of tumors.
Lymph flows in an open circuit from the tissues into lymphatic vessels, where it’s filtered by lymph nodes located throughout the body before being returned to the cardiovascular system. Unlike blood, which flows in a loop, lymph flows in only one direction. It’s not pumped like how blood is pumped by the heart, either—lymph flows passively, aided by other body movements like deep breathing and the action of nearby muscles and blood vessels. (That’s why we wear compression garments as a part of lymphedema treatment, as they help push the lymph along.)
When a lymphatic vessel has been damaged or compromised by things like surgery, injury or trauma, or improper development, lymph can’t drain from nearby tissues and flow through the body the way that it’s meant to. Instead, fluid builds up in these tissues, causing swelling. This is lymphedema at its simplest description: a chronic swelling.
Types of Lymphedema
There exists two types of lymphedema—primary and secondary.
Primary Lymphedema is caused by malformations of the lymphatic system, either through hereditary or congenital influences, and can either be present at birth or expressed later in life, such as during puberty or pregnancy. It is most common in the legs, but may also occur in the arms or torso. The National Institutes of Health (NIH) states that the incidence of primary lymphedema could be as high as 1 in 300 live births.
Secondary Lymphedema results from damage to the lymphatic system through traumatic injuries, surgical procedures, infections, etc. Secondary lymphedema occurs most commonly in the arms but can also develop in the legs. The World Health Organization estimates that over 150 million people worldwide have secondary lymphedema.
In the United States, nearly all cases of secondary lymphedema are cancer-related. This is due to surgical procedures such as mastectomies, lumpectomies with radiation, and the removal of lymph nodes. In fact, the Centers for Disease Control and Prevention (CDC) warns that the incidence of secondary lymphedema among cancer survivors is between 20-40%. Lympehdema is frequently associated with breast cancer, prostate cancer, pelvic area cancers, lymphoma, and melanoma; breast cancer is the most common cause of cancer-related lymphedema.
Stages of Lymphedema
Lymphedema is a chronic and oftentimes progressive condition, and so a staging system established by the International Society of Lymphology (ISL) is used for identifying the progression and severity of the condition. It is based on details about the amount of swelling and the condition of the tissues and skin. When the lymphedema is identified by stage, it makes it easier for a doctor or lymphedema therapist to accurately diagnose and prescribe treatment to improve, control, and monitor the patient’s swelling effectively.
A quick note about the staging system: despite the occurrence of lymphedema in other parts of the body, the staging system applies mostly to affected limbs.
Stage 0 is the “latent” or “preclinical” stage when there is a risk of developing lymphedema but no swelling is visible. Risk can be assessed through a physical exam, an MRI, lymphoscintigraphy, or bioimpedance (a non-invasive method of assessing the composition of body tissues). At this stage, it’s possible to prevent the lymphedema from developing further through immediate specialized treatment.
Stage I is when the affected limb is swollen and feels heavy, and pressing on the swollen area leaves a “pit” (dent). Symptoms worsen during the day, but temporarily resolve with elevation. It’s important to begin treatment as soon as these early signs are detected! Prompt treatment at this stage can help control and manage the condition, and may prevent it from becoming more severe.
Stage II occurs when pitting edema has progressed and changes in the tissues have begun. The limb is swollen but feels spongy, and, if fibrosis has developed, the limb will feel hard. The tissue changes happening at this stage increase the risks of even greater swelling, fibrosis, infections, and skin problems; elevation won’t reduce swelling, but intense treatment can usually improve Stage II lymphedema.
Stage III is the most advanced stage, and is also known as lymphostatic elephantiasis. The tissue has become extremely swollen and thickened, and infections are more common due to increased risks of skin breaks. A number of changes to the skin may occur, including changes in elasticity, color, and thickness. Intense therapy can potentially improve Stage III lymphedema and prevent it from worsening but it’s rare that it’s reversed to an earlier stage.
It’s important to seek treatment at the first signs of lymphedema because early diagnosis and treatment can help improve not only the prognosis but the condition, too. Although there’s no cure for lymphedema, there exists a variety of treatments to help manage swelling.
Tomorrow’s post will detail different treatment methods, so be sure to check back!
Support World Lymphedema Day
There are a number of ways you can help support World Lymphedema Day and show your solidarity! In addition to signing and sharing the petition, you can add a banner to your social media profile photos, or post your support on Twitter. These seemingly small gestures can inspire meaningful conversation about lymphedema and the need for awareness!
You can also support lymphatic research by donating to one of the research organizations, such as the Lymphatic Education & Research Network or the National Lymphedema Network, or by getting involved in the upcoming Lymphedema Lobby Days in D.C. this April.
Check out last year’s post for even more ideas on how to promote lymphedema awareness!
Keep checking back for more helpful information about lymphedema and lymphedema awareness throughout the week as we get closer to establishing the first-annual World Lymphedema Day on March 6th!
Thank you for sharing this. This is like my story. My legs were swelling my whole life and only at age of 25 founded out that it was lymphedema
Unfortunately the doctor did not say how is this possible, why could this be. I had to found out that it runs in my grandmother’s family but they all had different types of limb illnesses.
Thanks for your comment, and for sharing your story! I can totally relate: I wasn’t diagnosed until I was almost fifteen, and it’s frustrating to think of all those years of treatment I missed out on just because my doctors had no idea what lymphedema was. Considering that there are millions of people living with lymphedema worldwide, it’s baffling that so many people remain undiagnosed and untreated.
Hopefully things like the upcoming World Lymphedema Day will help change this. With awareness comes research, and with research comes more treatment options, earlier diagnoses, and better insurance coverage. We need to keep hoping— and keep fighting! :)
i was diagnosed last year at age 50 never had any swelling until i had total knee replacement in 2012 and got celluitius thats when it all began been to dr today and they tell me its edema sent me for stockings but i do get indentations when u press as the day wears on legs get tight and a heavy feelin and toes feeln theve been trampled on with an elephant so im no nearer what ive got.
Great post here. Not sure exactly how I came across your site, but glad I did. Will definitely be coming back.
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